Navigation Links
New model of muscular dystrophy provides insight into disease development
Date:8/27/2012

Muscular dystrophy is a complicated set of genetic diseases in which genetic mutations affect the various proteins that contribute to a complex that is required for a structural bridge between muscle cells and the extracellular matrix (ECM) that provides the physical and chemical environment required for their development and function. The affects of these genetic mutations in patients vary widely, even when the same gene is affected. In order to develop treatments for this disease, it is important to have an animal model that accurately reflects the course of the disease in humans. In this issue of the Journal of Clinical Investigation, researchers at the University of Iowa report the development of a mouse model of Fukuyama's muscular dystrophy that copies the pathology seen in the human form of the disease.

By removing the gene fukutin from mouse embryos at various points during development, researchers led by Kevin Campbell were able to determine that fukutin disrupts important modifications of dystrophin that prevent the muscle cells from attaching to the ECM. Disruption of the gene earlier in development led to a more severe form of the disease, suggesting that fukutin is important for muscle maturation. Disruptions in later stages of development caused a less severe form of the disease. In a companion piece, Elizabeth McNally of the University of Chicago discusses the implications of this disease model for the development of new therapies to treat muscular dystrophy.


'/>"/>
Contact: Jillian Hurst
press_releases@the-jci.org
Journal of Clinical Investigation
Source:Eurekalert

Page: 1

Related biology technology :

1. Isis And Collaborators Alleviate Disease In Animal Models Of Myotonic Dystrophy
2. NDIA, NTSA Will Be On Hand At Seventh Annual Capitol Hill Modeling and Simulation Expo, Set To Focus On Homeland Security, First Response
3. New life sciences R&D model based on real-time learning and data-sharing needed to sustain innovation
4. A new imaging system produces 3-D models of monuments using unmanned aircraft
5. MultiCell Technologies Announces Positive Preclinical Results for MCT-465 and MCT-485 in Primary Liver Cancer In Vitro Models
6. Newly-Published Study Compares SURFAXIN LS™ and CUROSURF® in Well-Established Model of Respiratory Distress Syndrome
7. Ahram Biosystems Announces Launch of the New Economy Model F1-12 of Its Palm PCR Series
8. SPX-106T Arrests Development of Atherosclerotic Plaques in Mouse Model of Cardiovascular Disease
9. Karyopharm Therapeutics Announces Multiple Presentations on Selective Inhibitors of Nuclear Export (SINE) in Solid and Hematologic Malignancy Models and Spontaneous Canine Cancers at the American Association of Cancer Research (AACR) Meeting
10. New Software from SRI Speeds Development of Genome-Scale Models for Drug and Fuel Research
11. A mouse model brings new perspectives on Lafora disease
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/23/2016)... ON (PRWEB) , ... June 23, 2016 , ... STACS ... DNA Technical Leader at the Arkansas State Crime Laboratory, has joined STACS DNA as ... the STACS DNA team,” said Jocelyn Tremblay, President and COO of STACS DNA. “In ...
(Date:6/23/2016)... , June 23, 2016 Apellis ... Phase 1 clinical trials of its complement C3 ... single and multiple ascending dose studies designed to ... (PD) of subcutaneous injection in healthy adult volunteers. ... (SC) either as a single dose (ranging from ...
(Date:6/23/2016)... Andrew D Zelenetz ... Published recently in Oncology ... touchONCOLOGY, Andrew D Zelenetz , discusses the ... is placing an increasing burden on healthcare systems ... With the patents on many biologics expiring, interest ...
(Date:6/23/2016)... ... June 23, 2016 , ... ... at the Pennsylvania Convention Center and will showcase its product’s latest features from ... also be presenting a scientific poster on Disrupting Clinical Trials in The Cloud ...
Breaking Biology Technology:
(Date:4/14/2016)... April 14, 2016 BioCatch ... Detection, today announced the appointment of Eyal Goldwerger ... role. Goldwerger,s leadership appointment comes at a ... of the deployment of its platform at several of ... technology, which discerns unique cognitive and physiological factors, is ...
(Date:3/31/2016)...  Genomics firm Nabsys has completed a financial  restructuring ... , M.D., who returned to the company in October ... team, including Chief Technology Officer, John Oliver , ... and Vice President of Software and Informatics, Michael ... Dr. Bready served as CEO of Nabsys from 2005-2014 ...
(Date:3/22/2016)... , March 22, 2016 ... report "Electronic Sensors Market for Consumer Industry by Type ... Others), Application (Communication & IT, Entertainment, Home ... Global Forecast to 2022", published by MarketsandMarkets, ... expected to reach USD 26.76 Billion by ...
Breaking Biology News(10 mins):